The many facets of Kaposi sarcoma-associated herpesvirus/human herpes virus-8 multicentric Castleman disease: clinical spectrum, diagnostic innovations, and therapeutic management

BACKGROUND: Castleman diseases encompass three distinct entities: unicentric Castleman disease, KSHV/HHV-8-associated multicentric Castleman disease (MCD), and idiopathic MCD. HHV-8 MCD is a life-threatening B-cell lymphoproliferative disorder driven by KSHV/HHV-8 infection of B cells, resulting in systemic inflammation and diverse clinical phenotypes. OBJECTIVE: To summarize current knowledge of HHV-8 MCD pathogenesis, clinical presentations, autoimmune complications, and diagnostic advances. METHODS: This review synthesizes existing literature on HHV-8 MCD, with particular attention to emerging diagnostic techniques, including liquid-phase detection of circulating viroblasts. RESULTS: Treatment approaches center on rituximab-based regimens, with outcomes varying between HIV-positive and HIV-negative populations. Novel diagnostic tools combining flow cytometry and viral transcript detection offer rapid identification of disease flares. CONCLUSION: These emerging diagnostic approaches may improve management of HHV-8 MCD, particularly in emergency settings where rapid flare identification is critical.